Clinical characteristics and prognostic risk factors of congenital pulmonary artery sling in children / 中华实用儿科临床杂志

Objective:To analyze the clinical and imaging characteristics of pulmonary artery sling (PAS) in children and to explore its diagnosis and treatment strategies and risk factors.Method:s A retrospective study was performed in 98 pediatric patients with PAS who were admitted to Guangzhou Women and Children′s Medical Center, Guangzhou Medical University, from January 2011 to October 2018.All their clinical features, imaging examination, treatment and prognosis were reviewed and analyzed.Result:s (1) Among the 98 patients with PAS, 56 cases were male and 42 cases were female.The age range of onset was 0-19 months with the median age of 2 months, and the age range of diagnosis was 0-84 months with the median age of 4 months.(2) The main clinical manifestations were cough (61/98 cases, 62.24%), recurrent wheezing(47/98 cases, 47.96%), shortness of breath (43/98 cases, 43.88%), cyanosis (19/98 cases, 19.39%), and laryngeal stridor (8/98 cases, 8.16%) and so on.(3) Ninety-six patients underwent cardiac ultrasonography, and the diagnostic rate was 87.50%(84/96 cases), and 62 cases (62/96 cases, 64.58%) of them were found to be associated with other congenital cardiovascular anomalies, among which atrial septal defect was the most common (32/96 cases, 33.33%). (4) Chest CT and three-dimensional reconstruction were performed in 92 PAS children, and the diagnostic rate was 100.00%.Airway stenosis was mainly in the middle and lower sections of the main bronchus (76/92 cases, 82.61%), and bronchobridge was found in 13 cases (13/92 cases, 39.13%). Bronchoscopy was performed in 77 children with PAS, showing complete tracheal cartilage ring in 43 cases (43/77 cases, 55.84%), and tracheobronchomalacia in 13 cases (13/77 cases, 16.88%). (5) Among 67 cases who had received left pulmonary artery (LPA) reimplantation, 5 cases died after operation because of airway stenosis and weaning failure (LPA reconstruction was performed in 1 case, and both LPA reconstruction and tracheal intervention were performed in the other 4 cases), 24 cases lost to follow-up, 38 cases survived, and the respiratory symptoms were improved in 2 to 96 months of follow-up.There were 31 cases of non-surgical treatment, of which 18 cases died of respiratory failure, 7 cases lost to follow-up, and the rest 6 cases showed respiratory symptoms of different degrees in the follow-up period.(6) Multivariate regression analysis showed that conservative treatment was an independent risk factor affecting the prognosis of PAS ( OR=7.45, 95% CI: 1.23-48.68). Conclusions:The combination of cardiac ultrasound, chest CT and bronchoscopy is important to the diagnosis of PAS.LPA reconstruction is the main method to treat PAS, which can improve respiratory symptoms, but its fusion with tracheal intervention poses a high risk of death.Conservative treatment is an independent risk factor that affects the prognosis of PAS children.

A review on progress of diagnosis and respiratory support therapy of acute respiratory distress syndrome / 中国中西医结合急救杂志

Acute respiratory distress syndrome (ARDS) is a clinical syndrome characterized by progressive hypoxemia and respiratory distress, which occurs in the course of critical illness, and diffuse interstitial and alveolar edema caused by injury of alveolar capillary endothelial cells and alveolar epithelial cells, the overall morbidity and mortality of ARDS are still high. In recent years, with a better understanding of ARDS, more and more researches on this subject have been made, establishing a new concept for the diagnosis and treatment of ARDS. Early respiratory function support and correction of hypoxia are the main measures for treatment of ARDS, and mechanical ventilation is still a primary method for respiratory support of ARDS patients. In this review, we present a summary of the modern advance in diagnosis and respiratory support for treatment of ARDS.

Serum vancomycin concentration distribution and its clinical therapeutic effect on children with severe Gram-positive cocci pneumonia / 临床儿科杂志

Objective To analyze the characteristics of serum vancomycin concentrations and its clinical therapeutic effects. Methods Serum vancomycin concentrations of 59 children diagnosed with severe Gram positive cocci pneumonia and treated with vancomycin were retrospectively analyzed. Vancomycin concentrations, biochemical values and disease status of patients were analyzed. Results The serum vancomycin concentrations of severe Gram positive cocci pneumonia children accompanied by acyanotic congenital heart disease was significantly higher than those without congenital heart disease, ( 12 . 12 mg/L vs 7 . 76 mg/L, P=0 . 008 ). The therapeutic effect of 40-60 mg/(kg·d) dosage group was signiifcantly higher than that of?60 mg/(kg·d) dosage group. Acute liver function damage and moderate/severe anemia may be risk factors for poor therapeutic effects to severe Gram positive cocci pneumonia children (P?

Clinical diagnosis, treatment, and prognosis of pulmonary artery sling in 38 children / 临床儿科杂志

Objective To explore the clinical characteristics and prognosis of congenital pulmonary artery sling (PAS) in children. MethodsThe clinical data of 38 children diagnosed with PAS during June 2009 and February 2015 were retrospectively analyzed. ResultsIn 38 PAS children, 35 cases (89.47%) were hospitalized for varying degrees of respiratory manifestations with recurrent cough (89.47%) and wheezing (84.21%) being the most common. The remaining 3 cases were found abnormal in routine preoperative examination and the diagnosis was confirmed after further examination. All 38 children were performed computer tomography angiography (CTA). Thirty-seven cases were diagnosed of PAS and diagnostic rate was 97.37%. One case was suspected of pulmonary dysplasia and diagnosed of PAS after operation. Twenty-six children received surgical treatment, of whom 25 children had pulmonary artery reconstruction (LPA). Seven children died during/after operation and 18 survived. The remaining 12 children received non-surgical treatment, of whom 9 died and 3 survived.ConclusionCardiac uhrasonography may reveal PAS in the early stage, while CTA is the best method for conifrmed diagnosis. LPA reconstruction is an important means of relieving left pulmonary artery oppression.

Study on three common mitochondrial DNA mutations in Leber's hereditary optic neuropathy / 中华医学遗传学杂志

<p><b>OBJECTIVE</b>To screen for genetic mutations in 35 patients with Leber's hereditary optic neuropathy (LHON).</p><p><b>METHODS</b>Polymerase chain reaction and DNA sequencing were used to screen for the presence of mitochondrial DNA mutations.</p><p><b>RESULTS</b>The total detection rate of top 3 common LHON mutations were 20.0%, which included 6 cases of ND4 11778 G to A, 1 case of ND1 3460 G to A. No ND6 14484 T to C mutation was detected. A ND4 G11719A synonymous mutation was found in all patients. In addition, 21 other mutations were discovered among 23 patients, among which 13 had a single mutation, 8 had a second mutations, and 2 had a third mutation. Among the 21 mutations, ND4 11778 G to A had a frequency of 28.6%(6/21). ND1 3552 T to A, ND6 14470 T to C, ND4 11794 T to C, ND1 3497 C to T and 3644 T to C respectively had a frequency of 19.0% (4/21), 19.0%(4/21), 14.3%(3/21), 9.5%(2/21) and 9.5%(2/21). Among the 3 patients who harbored a ND4 11794 T to C mutation, 2 were heteroplasmic and one was homoplasmic in nature.</p><p><b>CONCLUSION</b>The ND4 11778 G to A mutation is common in the Top "3" primary mutations of patients with LHON. Candidate LHON mutation ND1 3552 T to A or ND1 3644 T to C resulted in LHON pathogenesis as single or synergistic effect. The visual impairment at onset of the disease with candidate mutation were better than the eyes with the ND4 11778 G to A mutation.</p>

VEGF gene transfer modulates MMPs expression after vascular balloon injury / 中国病理生理杂志

AIM: To investigate the effect of VEGF gene transfer on matrix metalloproteinases(MMPs) and tissue inhibitor of metalloproteinases(TIMPs) expression after vascular balloon injury. METHODS: 90 New Zealand white rabbits were divided into 3 groups randomly: group Ⅰ(balloon injury group), groupⅡ (pAdtrackCMV group) and group Ⅲ(pAdtrackCMV-VEGF165 group). Hypercholesterol diet was given for 7 days before experiment and continued to receive until the animals were killed. Each group were divided into five subgroups according to the sacrifice time. Blood samples and iliac arteries were harvested for further analysis. RESULTS: In group Ⅰ and Ⅱ, MMP2 and TIMP1,2 expressions were detected during the whole process ,while in group III ,MMP1,2,9 and TIMP1,2 could be detected from 3 days after gene transfer and reached the highest level at 1 week and could not be detected at 8 week. CONCLUSIONS: Imbalance expression of MMPs and TIMPs occurred after vascular injury and this may be the reason of pathological remodeling. Local phAdtrackCMV-VEGF165 transfer could specifically change the expression of MMPs and facilitate the positive remodeling process after vascular injury.